AI Article Synopsis
- Lynch syndrome (LS) is linked to a higher risk of various tumors, including colorectal and non-colorectal cancers, with recent studies suggesting a potential association with sarcomas.
- A systematic review of 44 studies involving LS patients revealed that most sarcomas developed in individuals with a mutation in the MSH2 gene and showed distinct genetic features typical of LS-related tumors.
- Among the types of sarcomas observed, undifferentiated pleomorphic sarcoma, leiomyosarcoma, and liposarcoma were the most common, but there was also a notable occurrence of rhabdomyosarcoma, warranting further research to understand this specific group better.
Article Abstract
Lynch syndrome (LS) is a genetic predisposition leading to colorectal and non-colorectal tumors such as endometrial, upper urinary tract, small intestine, ovarian, gastric, biliary duct cancers and glioblastoma. Though not classically associated with LS, growing literature suggests that sarcomas might develop in patients with LS. This systematic review of literature identified 44 studies (N = 95) of LS patients who developed sarcomas. It seems that most sarcomas developed in patients with a germline mutation of MSH2 (57 %) exhibit a dMMR (81 %) or MSI (77 %) phenotype, as in other LS-tumors. Although undifferentiated pleomorphic sarcoma (UPS), leiomyosarcoma, and liposarcoma remain the most represented histologic subtype, a higher proportion of rhabdomyosarcoma (10 %, especially pleomorphic rhabdomyosarcoma) is reported. Further studies are required to better characterize this sub-population.
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| http://dx.doi.org/10.1016/j.critrevonc.2023.104055 | DOI Listing |
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